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Progressive ventricular dysfunction is not uncommon in patients with univentricular hearts as they age. In the acute setting vasoactive support can be employed, but is not always sufficient and patients occasionally require mechanical support. We report the successful implantation and subsequent challenges of a percutaneous Abiomed Impella ventricular assist device as a rescue therapy for a 15-year old-patient with Fontan circulation and severe ventricular dysfunction after cardiac arrest.

The accuracy of echocardiography in evaluating left ventricular contractility has not been validated in children. The objective of this study was to compare echocardiographic measures of contractility with those derived from pressure-volume loop (PVL) analysis in children.Patients with relatively normal loading conditions undergoing routine left heart catheterization were prospectively enrolled. PVLs were obtained via conductance catheters. The gold-standard measure of contractility, end-systolic elastance (Ees), was obtained via balloon occlusion of one or both vena cavae. Echocardiograms were performed immediately after PVL analysis under the same anesthetic conditions. Single-beat estimations of echocardiographic Ees were calculated using four different methods. These estimates were calculated using a combination of noninvasive blood pressure readings, ventricular volumes derived from three-dimensional echocardiography, and Doppler time intervals.Of 24 patients, 18 patients were heart transplant recipients, and six patients had small patent ductus arteriosus or small coronary fistulae. The mean age was 9.1 ± 5.6 years. The average invasive Ees was 3.04 ± 1.65 mm Hg/mL. Invasive Ees correlated best with echocardiographic Ees by the method of Tanoue (r = 0.85, P .01), with a mean difference of -0.07 mm Hg/mL (95% limits of agreement, -2.0 to 1.4 mm Hg/mL).Echocardiographic estimates of Ees correlate well with gold-standard measures obtained via conductance catheters in children with relatively normal loading conditions. The use of these noninvasive measures in accurately assessing left ventricular contractility appears promising and merits further study in children.

Sildenafil has been reported to improve exercise capacity in Fontan patients, but the physiologic mechanisms behind these findings are not completely understood. The objective of this study was to study the acute effect of sildenafil on pressure–volume loop (PVL) measures of ventricular function in Fontan patients. Patients after Fontan operation who were presenting for a clinically indicated catheterization were enrolled. Patients were randomized in a double-blinded fashion to receive placebo (n = 9) or sildenafil (n = 10) 30–90 min prior to catheterization. PVLs were recorded using microconductance catheters at baseline and after infusion of dobutamine (10 mcg/kg/min). The primary outcome was change in ventriculoarterial (VA) coupling. For the entire cohort, VA coupling trended toward improvement with dobutamine (1.4 ± 0.4 to 1.8 ± 0.9, p = 0.07). End-systolic elastance showed improvement (2.6 ± 0.9 to 3.8 ± 1.4 mmHg m2/ml, p

Background Low superior vena cava (SVC) blood flow has recently been identified as a marker for bidirectional superior cavopulmonary connection (SCPC) failure and death. Prior innominate vein occlusion is considered a significant anatomic risk factor for SCPC failure. We therefore evaluated the outcomes of infants who underwent SCPC with known upper-body venous obstruction. Methods Between February 1995 and June 2014, SCPC was carried out in 8 patients who had either a single SVC with known prior occlusion of the innominate vein (n = 6) or bilateral SVCs without a bridging vein with occlusion of one SVC (n = 2). The cause of the occlusion was an indwelling catheter in 5 patients. These patients were compared with 8 patients with normal upper-body venous drainage who underwent SCPC. Patients were evaluated for preoperative risk factors (including SVC size, pulmonary artery size, Nakata index, pulmonary vascular resistance), operative factors, and clinical outcomes to determine the impact of prior upper-body venous occlusion on SCPC failure or death. Results There were no significant differences in preoperative risk factors between the two groups, except for a significantly lower Nakata index in the study group with a trend toward smaller pulmonary artery branch size. There were no SCPC takedowns or mortalities. There was no significant difference in postoperative length of stay (median of 7 days [range, 5 to 32 days] versus 5 days [range, 4 to 32 days]; p = 0.17. Study patients had a lower mean systemic oxygen saturation at discharge, 81% versus 85% ( p = 0.05). In the study group, at a median follow-up of 42 months, 3 patients underwent successful Fontan completion and 5 are still awaiting Fontan completion. Conclusions Although patients with prior upper-body venous obstruction may have lower systemic oxygen saturations at hospital discharge, they do not demonstrate an increased SCPC failure or mortality rate. Innominate vein occlusion or its equivalent in patients with bilateral SVCs should not preclude the performance of SCPC. Physiologic rather than anatomic evaluation of preoperative systemic venous return may be more useful to predict outcome after SCPC.

BACKGROUND: Pediatric heart transplant recipients are at risk for increased LV diastolic stiffness. However, the non-invasive evaluation of LV stiffness has remained elusive in this population. The objective of this study was to compare novel echocardiographic measures of left ventricular (LV) diastolic stiffness vs. gold-standard measures derived from pressure-volume loop (PVL) analysis in pediatric heart transplant recipients. METHODS: Patients undergoing left heart catheterization were prospectively enrolled. PVLs were obtained via conductance. The end-diastolic pressure-volume relationship was obtained via balloon occlusion. β, the stiffness constant, was calculated. Echocardiographic measures of diastolic function were derived from spectral and tissue Doppler and 2D speckle-tracking. Ventricular volumes were measured by 3D echocardiography. The novel echocardiographic estimates of ventricular stiffness included E:e′/EDV and E:early diastolic strain rate/EDV. RESULTS: Of 24 children, 18 were heart transplant recipients. Six control patients had a hemodynamically insignificant patent ductus arteriosus or coronary fistula. Mean age was 9.1±5.6 years. Median EDP was 9 mmHg (IQR 8–13 mmHg). Lateral E:e′/EDV (r=0.59, p0.015 mL(−1)). A lateral E:e′/EDV of >0.15 mL(−1) had an 89% sensitivity and 93% specificity in detecting an abnormal β. CONCLUSION: Echocardiographic estimates of ventricular stiffness may be accurate when compared to the gold-standard in pediatric heart transplant recipients. The clinical usefulness of these non-invasive measures in assessing LV stiffness merits further study in children.

AIMS: The relationship between echocardiographic measures of left ventricular (LV) systolic function and reference-standard measures have not been assessed in children. The objective of this study was to assess the validity of echocardiographic indices of LV systolic function via direct comparison to a novel composite measure of contractility derived from pressure–volume loop (PVL) analysis. METHODS AND RESULTS: Children with normal loading conditions undergoing routine left heart catheterization were prospectively enrolled. PVLs were obtained via conductance catheters. A composite invasive composite contractility index (ICCI) was developed using data reduction strategies to combine four measures of contractility derived from PVL analysis. Echocardiograms were performed immediately after PVL analysis under the same anesthetic conditions. Conventional and speckle-tracking echocardiographic measures of systolic function were measured. Of 24 patients, 18 patients were heart transplant recipients, 6 patients had a small patent ductus arteriosus or small coronary fistula. Mean age was 9.1 ± 5.6 years. Upon multivariable regression, longitudinal strain was associated with ICCI (β = −0.54, P = 0.02) while controlling for indices of preload, afterload, heart rate, and LV mass under baseline conditions. Ejection fraction and shortening fraction were associated with LV mass and load indices, but not contractility. CONCLUSION: Speckle-tracking derived longitudinal strain is associated ICCI in children with normal loading conditions. Longitudinal measures of deformation appear to accurately assess LV contractility in children.

Hybrid Norwood palliation is gaining acceptance as an alternative to open Norwood palliation in high-risk neonates with hypoplastic left heart syndrome. Aortic dissection after hybrid Norwood palliation is a rare but ominous complication that has not been reported.

Objective To compare the interstage cardiac catheterization hemodynamic and angiographic findings between shunt types for the Pediatric Heart Network Single Ventricle Reconstruction trial. The trial, which randomized subjects to a modified Blalock-Taussig shunt (MBTS) or right ventricle-to-pulmonary artery shunt (RVPAS) for the Norwood procedure, demonstrated the RVPAS was associated with a smaller pulmonary artery diameter but superior 12-month transplant-free survival. Methods We analyzed the pre–stage II catheterization data for the trial subjects. The hemodynamic variables and shunt and pulmonary angiographic data were compared between shunt types; their association with 12-month transplant-free survival was also evaluated. Results Of 549 randomized subjects, 389 underwent pre–stage II catheterization. A smaller size, lower aortic and superior vena cava saturation, and higher ventricular end-diastolic pressure were associated with worse 12-month transplant-free survival. The MBTS group had a lower coronary perfusion pressure (27 vs 32 mm Hg; P P = .009). A greater pulmonary blood flow/systemic blood flow ratio increased the risk of death or transplantation only in the RVPAS group ( P = .01). The MBTS group had fewer shunt (14% vs 28%, P = .004) and severe left pulmonary artery (0.7% vs 9.2%, P = .003) stenoses, larger mid-main branch pulmonary artery diameters, and greater Nakata indexes (164 vs 134, P Conclusions Compared with the RVPAS subjects, the MBTS subjects had more hemodynamic abnormalities related to shunt physiology, and the RVPAS subjects had more shunt or pulmonary obstruction of a severe degree and inferior pulmonary artery growth at pre–stage II catheterization. A lower body surface area, greater ventricular end-diastolic pressure, and lower superior vena cava saturation were associated with worse 12-month transplant-free survival.

Traumatic aortic injuries occur rarely in the pediatric population. Adult vascular interventionalists more frequently encounter aortic injury, and endovascular graft placement is a commonly used management approach. The standard of care in pediatric traumatic aortic injury is an open surgical repair, although it is not always the optimal approach. Endovascular graft placement has been trialed in pediatric patients, but its use has technical limitations. We describe the case of an 8 year old female passenger in a motor vehicle collision, resulting in formation of a traumatic aortic pseudoaneurysm. Due to her co-morbidities, she was a high risk surgical candidate, and therefore, underwent successful percutaneous implantation of an investigational balloon-expandable covered Cheatham platinum (CP) stent (NuMed, Inc., Hopkinton, New York). This case demonstrates the utility of balloon-expandable covered stents for treatment of pediatric traumatic aortic injury (TIA) as an alternative to open surgical repair or percutaneous endograft implantation.

Three-dimensional (3D) transesophageal echocardiography (TEE) has numerous potential applications in the care of patients with congenital heart disease (CHD). However, there were few data examining its utility in this setting. The aim was to describe the initial experience and feasibility of this modality at a tertiary CHD center. Twenty-seven 3D-TEE studies using the x7-2t live 3D matrix TEE transducer with an iE33 echocardiographic scanner (Philips Medical Systems, Bothell, Washington) were attempted. The utility of this method was examined in the 3 settings of interventional catheterizations (n = 16), intraoperative studies (n = 4), and diagnostic studies (n = 7). The probe was successfully inserted in 26 of 27 patients (weight 22.6 to 110 kg). In conclusion, the current matrix-array 3D-TEE probe was found to have a wide variety of clinical applications in a CHD center.

Background Traditional palliation of infants with biventricular hearts and inadequate pulmonary blood flow is a modified Blalock-Taussig shunt. The aim of this report is to assess the results of an alternative, right ventricle-to-pulmonary artery (RV-PA) shunt. Methods Between August 2004 and July 2007, 10 infants with biventricular hearts and inadequate pulmonary blood flow underwent palliation with an RV-PA shunt. Median age was 9 days (range, 4 to 86), weight was 3.0 kg (1.7 to 4.5), and 4 of 10 patients weighed less than 2.5 kg. Shunts were nonvalved Gore-Tex (W.L. Gore Assoc, Flagstaff, AZ), and size was 6 mm (n = 5) or 5 mm (n = 5). Results There were no operative deaths. Median oxygen saturation at hospital discharge was 95% (87 to 98). In 2 patients the shunt was partially narrowed with a metal clip; they underwent successful balloon dilation 6 months after shunt placement. Eight patients have undergone two-ventricle repair 6 to 17 months after shunt placement. At the time of complete repair, oxygen saturation was 86 ± 1% and weight was 7.7 ± 1.7 kg. Repairs included a valved RV-to-PA conduit, 14 to 16 mm in diameter. There was one interstage death. Conclusions The RV-PA shunt provides successful palliation in infants with biventricular heart disease and inadequate pulmonary blood flow. It can be used in low birth weight infants and allows significant growth with protection of oxygen saturation prior to complete repair. Partial clipping of the shunt with subsequent balloon dilation is an option to prolong palliation. These results compare favorably with those of a modified Blalock-Taussig shunt or single stage complete repair.

Introduction: A recent modification to the Norwood procedure involving a shunt placed directly from the right ventricle to the pulmonary arteries may improve postoperative haemodynamics. Concerns remain, however, about the potential problems produced by the required ventriculotomy. Methods: We compared 76 patients with hypoplastic left heart syndrome who underwent the Norwood procedure, 35 receiving a modified Blalock-Taussig shunt and the remaining 41 a shunt placed directly from the right ventricle to the pulmonary arteries. We reviewed their subsequent progress through the second stage of palliation. A single observer graded right ventricular function, and the severity of tricuspid regurgitation, based on blinded review of the most recent echocardiograms prior to the second stage of palliation. Results: At the time of catheterization prior to the second stage, patients with a shunt placed from the right ventricle to the pulmonary arteries, rather than a modified Blalock-Taussig shunt, had higher arterial diastolic blood pressure, at 44 versus 40 millimetres of mercury, p equal to 0.02, lower ventricular end diastolic pressures, at 8 versus 11 millimetres of mercury, p equal to 0.0002, and larger pulmonary arteries as judged using the Nakata index, at 270 versus 188 millimetres squared per metres squared, p equal to 0.009. There was no difference in qualitative ventricular systolic function or tricuspid regurgitation between groups. No differences were found between groups during the hospitalization following the second stage of palliation. A trend towards improved survival to the second stage was seen following the construction of a shunt from the right ventricle to the pulmonary arteries. Conclusions: Construction of a shunt from the right ventricle to the pulmonary arteries is associated with lower right ventricular end diastolic pressures, larger pulmonary arterial size, and higher systemic arterial diastolic pressures. No apparent deleterious effects of the right ventriculotomy were observed in terms of qualitative ventricular systolic function or tricuspid regurgitation.

Introduction: The accuracy of echocardiography in evaluating left ventricular (LV) diastolic function has not been validated in children. The objective of this study was to compare echocardiographic and gold-standard measures of LV diastolic function in children. Methods: Patients undergoing routine left heart catheterization were prospectively enrolled. Pressure-volume loops (PVL) were obtained via conductance catheters. The end-diastolic pressure-volume relationship was obtained via balloon occlusion of the vena cavae. PVL measures of diastolic function were divided into early active relaxation (the isovolumic relaxation time constant, tau), and ventricular stiffness (the chamber stiffness constant, β). End-diastolic pressure (EDP) was also recorded. Echocardiographic measures of diastolic function were derived from spectral Doppler, tissue Doppler, and 2D speckle-tracking. The relationships between PVL and echocardiographic measures were determined using Spearman’s correlation. Results: Of 24 patients, 18 patients were s/p heart transplant, 5 patients had a small patent ductus arteriosus or coronary fistula. Mean age was 9.1 ± 5.6 years. The median τ was 24.9 ms (IQR 22.8 - 28.4 ms), median β was 0.094 (IQR 0.035 - 0.154), and median EDP was 9 mmHg (IQR 8 - 13 mmHg). Statistically significant correlations between invasive and echocardiographic measures of diastolic function are reported in the Table. No echocardiographic measures correlated with β. Conclusion: Early diastolic echocardiographic measures correlate with tau and may accurately represent early active relaxation in children. Modest associations exist between echocardiographic measures and EDP. The use of these non-invasive measures in accurately assessing LV diastolic function appears promising in children. However, no echocardiographic measures correlate with chamber stiffness. The development of such measures merits further study.

Neonates with hypoplastic left heart syndrome and high-risk factors for an open Norwood procedure may benefit from a hybrid stage I procedure. The presence of a giant patent ductus arteriosus prevents safe deployment of the ductus arteriosus stent. We describe a new technique that involves banding the patent ductus arteriosus, therefore allowing stent implantation during hybrid stage I palliation.

Pulmonary arteriovenous malformations (AVMs) large enough to lead to clinically significant cyanosis are rare in the pediatric population. To date, there has been some experience with transcatheter embolization of pulmonary AVMs in children, primarily with coils or balloons. Herein, we report 2 cases of children who were progressively symptomatic and had physical manifestations of hypoxemia arising from large pulmonary AVMs. Both improved after successful catheter-based placement of multiple occlusion devices (Amplatzer vascular plugs) in the pulmonary arterial segments feeding the AVMs produced a rapid, sustained increase in oxygen saturations, and a subsequent amelioration of their symptoms. This represents the first case series of multiple Amplatzer vascular plugs placed into numerous arteriovenous formations, exclusively in children. This approach represents an additional nonsurgical option for children or adults with symptomatic pulmonary AVMs.

Background Complete repair of conotruncal defects frequently uses maintenance of an interatrial communication. Postoperative right ventricular dysfunction may be characterized by elevated right atrial pressure and decreased systemic oxygen saturation owing to right-to-left shunting at the atrial level. Methods From January 1996 to December 2005, 112 patients younger than 6 months of age underwent complete repair of tetralogy of Fallot or truncus arteriosus. An interatrial communication was used in 80 of 112 patients (71%). Hemodynamic data were determined during the first 48 hours after surgery. Results In patients with an atrial communication, mean oxygen saturation reached a nadir of 94% ± 6%, and mean arterial Po 2 a nadir of 73 ± 25 mm Hg at 16 to 24 hours after surgery; both increased during the second 24 hours. At hospital discharge, median oxygen saturation was 98% (range, 86% to 100%). During the first 48 hours, mean oxygen saturation was less than 90% in 13 patients; the only multivariate risk factor was younger patient age. Mean right atrial pressure was greater than 10 mm Hg in 30 patients; multivariate risk factors were older patient age and repair with a transannular patch. Conclusions After complete repair of conotruncal defects using an interatrial communication, systemic oxygenation reaches a nadir at 24 hours after surgery, and improves by the time of hospital discharge. Clinically significant desaturation occurs in a small minority of patients. Infants undergoing repair before 2 months of age are at higher risk for systemic desaturation. The effects of an interatrial communication on systemic oxygenation should not be considered a contraindication to complete repair in early infancy.

Background: Prior studies suggest that patent foramen ovale (PFO) diameter >4 mm is associated with a high probability of cryptogenic ischemic stroke (CIS). Methods: We evaluated all patients diagnosed with CIS who underwent closure of intra-atrial communication (IAC) using the Amplatzer atrial septal defect (ASD) occluder in our institution between August 1997 and March 2004. For each IAC, echocardiographic diameters and balloon-stretched diameters were recorded. Stretchability index was calculated as the ratio of stretched diameter to unstretched diameter. Results: Fifty-six patients met the inclusion criteria for this study. There was an inverse logarithmic relationship between unstretched IAC diameter and stretchability index. For the 28 smaller defects, the median IAC diameter was 2 mm, and median stretchability index was 5.58 (range 2.6–15). For the 28 larger defects, median diameter was 6 mm, and median stretchability index was 2.38 (range 1.05–5). The difference in stretchability index between the two groups was significant (P < 0.0001). Conclusion: Our data bring into question the concept that the diameter of the defect would singularly predict the probability of stroke.

The aim of this study was to determine if preoperative propranolol therapy has a deleterious effect on postoperative variables in patients with tetralogy of Fallot. Data from 97 patients who underwent complete repair of tetralogy of Fallot were reviewed. The patients were divided into 2 groups: those receiving preoperative propranolol therapy (n = 32) and those not receiving therapy (n = 65). Preoperative and intraoperative variables did not differ between groups. There were no differences in postoperative inotrope scores on arrival to the intensive care unit and through the first 12 hours postoperatively. There was a trend toward increased inotrope scores at 24 hours (median 8 vs 5, p = 0.05) and 48 hours (median 8 vs 3, p = 0.05) postoperatively in the patients treated with propranolol. Temporary pacing in the early postoperative period occurred more often in the propranolol group (16% vs 3%, p = 0.04). There was no difference between groups in length of mechanical ventilation, intensive care unit stay, or total hospital postoperative stay. In conclusion, propranolol therapy can be used in patients with tetralogy of Fallot until the time of surgery, without important effects on their postoperative courses. Any blunting of inotropic or chronotropic activity in propranolol-treated patients appears to be easily overcome with increased inotropic medications or temporary pacing, without increased morbidity or mortality.

of an early aggressive treatment was life saving. Compared with the scheduled device implantation in patients deteriorating on the waiting list, management of emergencies requiring circulatory support is still a challenging situation. We have developed in our unit a mobile kit of emergency circulatory support to deal with this problem. This ECLS has become a useful adjunct to our cardiac assist device program, allowing patient transfer and secondary implantation of a more long-term sophisticated device. As expected in this catecholamine-induced cardiomyopathy, the patient recovered after surgical resection of the tumor, but recovery is not so fast as to allow the use of an ECLS device alone, and implantation of a long-term device was mandatory.

In patients with pulmonary hypertension, pulmonary vasodilator testing with inhaled nitric oxide (NO) during cardiac catheterization provides valuable data for defining future care plans. Previously, the use of delivery systems for spontaneously breathing individuals required a tight-fitting seal by face mask and an approved delivery and dilution device. We hypothesized that a simplified delivery system using nasal cannula could be utilized to effectively deliver NO during cardiac catheterization. We developed a simple delivery system to deliver through a nasal cannula a concentration of NO at 50 ppm at the nares along with supplemental oxygen (O2) via face tent. We prospectively employed this system for 10-minute intervals on 11 patients (age range, 7 months to 41 years) with pulmonary hypertension undergoing scheduled cardiac catheterization. Mean pulmonary artery pressure (PAp) decreased from 62 mmHg (range, 38-99) at room air testing to 45 mmHg (range, 36-91) with the addition of NO plus O2 (p = 0.014). Pulmonary vascular resistance (PVR) decreased from 11.6 U.m2 (range, 4.5-43.4) to 6.3 U.m2 (range, 2.0-34.2) (p = 0.001). A response of 20% or more reduction in PVR was seen in all 11 patients. The initial ratio of pulmonary to systemic vascular resistance (Rp:Rs) was 0.49 (range, 0.25-3.5) and decreased to 0.35 (range 0.1-2.6) (p = 0.002). No adverse side effects were noted. We found this NO delivery system to be a simple and effective method of pulmonary vasodilatory testing that may have wide applicability in the cardiac catheterization laboratory.

Background A modification to the Norwood procedure involving a right ventricle-to-pulmonary artery (RV-PA) shunt may improve early postoperative outcomes. Concerns remain about the effect of the right ventriculotomy required with this shunt on long-term ventricular function. Methods Between January 2000 and April 2005, 76 patients underwent the Norwood procedure, 35 with a modified Blalock-Taussig shunt (mBTS) and 41 with a RV-PA shunt. Patients were monitored until death or September 1, 2009, with an average follow-up of 6.8 years. Cardiac catheterization, echocardiograms, perioperative Fontan courses, and need for cardiac transplantation were compared between groups. Results Cumulative survival was 63% (22 of 35) in the mBTS group vs 78% (32 of 41) in the RV-PA group (p = 0.14). Pre-Fontan echocardiography revealed poorer ventricular function in RV-PA patients (p = 0.03). Cardiac transplantation was required in 6 of 32 (19%) patients with a prior RV-PA shunt vs 1 of 23 (4%) in the mBTS group (p = 0.06). This results in an almost identical cumulative transplant-free survival between groups; 60% (21 of 35) in the mBTS group and 63% (26 of 41) in the RV-PA group (p = 0.95). Conclusions Neither shunt offers a clear survival advantage through an average follow-up of 6.8 years. The RV-PA shunt results in impaired late ventricular function that may result in an increased need for cardiac transplantation.

The images and videos presented in this article illustrate a diagnostic transseptal left heart catheterization for a patient with a prosthetic mitral valve guided by live three-dimensional (3D) transesophageal echocardiography. This method provided high-quality 3D imaging that was useful in guiding transseptal puncture and demonstrating prosthetic valve function during this evaluation.

As the field of catheter-based therapies for congenital heart disease continues to expand, we lack the evidence-based data to make appropriate therapeutic decisions in the catheterization laboratory. A stumbling block to the determination of evidence-based therapies is our inability to simply and reliably share outcome data across multiple centers. We investigated whether a commonly used congenital heart disease catheterization database program (PedCath) could be used as an automatic catheterization data submission tool to a central database for outcome analysis. To test the feasibility of such a tool for collaborative outcomes research we formed a national group of seven congenital heart disease centers, the Mid-Atlantic Group of Interventional Cardiology, to warehouse and analyze catheterization data. We successfully modified PedCath to transfer the results of catheter-based therapies on 256 therapeutic procedures for atrial septal defect (ASD) closure, coarctation of the aorta angioplasty and stenting, and pulmonary and aortic balloon valvuloplasties over a 13-month pilot period. Short-term follow-up within the 13-month period was received on 31 patients. This study demonstrated the successful development of a simple process, requiring minimal data entry for investigators from around the world to share cardiac catheterization data for long-term outcome determination of catheter-based therapies for congenital heart disease.

Prior studies suggest that patent foramen ovale (PFO) diameter4 mm is associated with a high probability of cryptogenic ischemic stroke (CIS).We evaluated all patients diagnosed with CIS who underwent closure of intra-atrial communication (IAC) using the Amplatzer atrial septal defect (ASD) occluder in our institution between August 1997 and March 2004. For each IAC, echocardiographic diameters and balloon-stretched diameters were recorded. Stretchability index was calculated as the ratio of stretched diameter to unstretched diameter.Fifty-six patients met the inclusion criteria for this study. There was an inverse logarithmic relationship between unstretched IAC diameter and stretchability index. For the 28 smaller defects, the median IAC diameter was 2 mm, and median stretchability index was 5.58 (range 2.6-15). For the 28 larger defects, median diameter was 6 mm, and median stretchability index was 2.38 (range 1.05-5). The difference in stretchability index between the two groups was significant (P0.0001).Our data bring into question the concept that the diameter of the defect would singularly predict the probability of stroke.

Despite dramatic progress in neonatal cardiac surgery, prematurity and low birth weight remain risk factors for poor outcome. Attempts to delay intervention with supportive therapy have been shown to increase morbidity and mortality. We present a case of an 840 gram, 28-week gestation newborn with tetralogy of Fallot, in whom palliation was achieved with a right ventricular outflow tract stent. This management allowed subsequent successful complete repair.

Objective Prognostic factors for survival after bidirectional cavopulmonary anastomosis for functionally single ventricle are not well defined. We analyzed preoperative hemodynamic and echocardiographic data to determine risk factors for death or transplantation at least 1 year after bidirectional cavopulmonary anastomosis. Methods Data for all patients who underwent bidirectional cavopulmonary anastomosis before 5 years of age at our institution from September 1995 through June 2005 were analyzed. Available preoperative echocardiograms and catheterizations were reviewed. Survivors were compared with those who died or underwent transplantation. Bivariable associations between demographic and clinical risk factors and survival status (alive without transplantation vs dead or transplanted) were assessed with Wilcoxon rank sum test and χ 2 or Fisher exact tests. Survival functions were constructed with Kaplan–Meier estimates, and event times compared between subgroups with log–rank tests. Cox proportional hazard modeling was used for multivariable modeling of risk of death or transplantation. Results One hundred sixty-seven patients underwent bidirectional cavopulmonary anastomosis with hemi-Fontan (n = 62) or bidirectional Glenn (n = 105) operations. Three patients died before discharge, 11 died later, and 1 has undergone transplantation. Freedom from death or transplantation after bidirectional cavopulmonary anastomosis was 96% at 1 year and 89% at 5 years. Multivariable analysis of preoperative variables showed atrioventricular valve regurgitation to be an independent risk factor for death or transplantation (hazard ratio 2.8, 95% confidence interval 1.1–7.1, P = .02). Conclusion Although survival after bidirectional cavopulmonary anastomosis is high, preoperative atrioventricular valve regurgitation is an important risk factor for death or transplantation.

BACKGROUND Right ventricular endomyocardial biopsy is the gold standard for detecting active myocardial inflammation in cardiomyopathy as well as rejection after cardiac transplantation. This procedure has historically required the exclusive use of fluoroscopic guidance to guide catheter, sheath, and bioptome manipulation. The current study evaluates the feasibility and utility of real-time transthoracic three-dimensional echocardiography (3DE) to guide right ventricular endomyocardial biopsies in children. METHODS From July 2003 to April 2004, we utilized real-time 3DE in 28 consecutive cardiac catheterizations in children aged 18 months to 16 years who were undergoing endomyocardial biopsy. A commercially available 3DE scanner (Philips Sonos 7500) equipped with a 2-4 MHz 3D matrix array transthoracic probe was utilized in all cases. RESULTS A total of 123 endomyocardial biopsy samples were obtained in nine patients (BSA 0.85 m(2)+/- 0.33 m(2)). Of these 123 samples, 99 (80%) were obtained with the use of real-time transthoracic 3DE. There were no complications, including no new tricuspid valve leaflet flail or pericardial effusion. 3DE proved to be a reliable noninvasive modality to properly direct the bioptome to the desired site of biopsy within the right ventricle. As familiarity with this technique increased, the need for fluoroscopic guidance of bioptome manipulation in the right ventricle was minimized. CONCLUSIONS The use of real-time transthoracic 3DE in endomyocardial right ventricular biopsies in children is both feasible and safe. Further study to determine the impact of real-time 3DE guidance on fluoroscopy and case times for endomyocardial biopsies is warranted.